European cystic fibrosis society standards of care: Best practice guidelines

Alan R. Smyth, Scott C. Bell, Snezana Bojcin, Mandy Bryon, Alistair Duff, Patrick Flume, Nataliya Kashirskaya, Anne Munck, Felix Ratjen, Sarah Jane Schwarzenberg, Isabelle Sermet-Gaudelus, Kevin W. Southern, Giovanni Taccetti, Gerald Ullrich, Sue Wolfe

Research output: Contribution to journalReview articlepeer-review

452 Scopus citations

Abstract

Specialised CF care has led to a dramatic improvement in survival in CF: in the last four decades, well above what was seen in the general population over the same period. With the implementation of newborn screening in many European countries, centres are increasingly caring for a cohort of patients who have minimal lung disease at diagnosis and therefore have the potential to enjoy an excellent quality of life and an even greater life expectancy than was seen previously. To allow high quality care to be delivered throughout Europe, a landmark document was published in 2005 that sets standards of care. Our current document builds on this work, setting standards for best practice in key aspects of CF care. The objective of our document is to give a broad overview of the standards expected for screening, diagnosis, pre-emptive treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support. For comprehensive details of clinical care of CF, references to the most up to date European Consensus Statements, Guidelines or Position Papers are provided in Table 1. We hope that this best practice document will be useful to clinical teams both in countries where CF care is developing and those with established CF centres.

Original languageEnglish (US)
Pages (from-to)S23-S42
JournalJournal of Cystic Fibrosis
Volume13
Issue numberS1
DOIs
StatePublished - May 2014

Bibliographical note

Funding Information:
A.R. Smyth: personal fees from Gilead, other from MPEX, other from Pharmaxis, other from Vertex, grants from Forest Labs, outside the submitted work; S.C. Bell: grants and non-financial support from Vertex Pharmaceuticals, personal fees and other from Novartis, other from Gilead, personal fees and other from Rempex, outside the submitted work; P. Flume: grants and personal fees from Aptalis, grants and personal fees from Gilead Sciences, Inc, grants and personal fees from Bayer Healthcare AG, grants and personal fees from Novartis, grants and personal fees from Vertex Pharmaceuticals, Inc, grants and personal fees from Pharmaxis Limited, grants from Boehringer Ingelheim Pharmceuticals, grants from Grifols, grants from Savara Pharma, grants from KaloBios, grants from Cystic Fibrosis Foundation, grants from National Institutes of Health, grants and personal fees from Genentech, outside the submitted work; A. Munck: personal fees from Vertex, Novartis, personal fees from Vertex, outside the submitted work; F. Ratjen: personal fees from Boehringer Ingelheim, during the conduct of the study; personal fees from Vertex, personal fees from Novartis, personal fees from Bayer, personal fees from Talecris, personal fees from CSL Behring, personal fees from Roche, personal fees from Gilead, grants from Novartis, personal fees from Genetech, personal fees from Genetech, personal fees from Pari, outside the submitted work; S.J. Schwarzenberg: consulting for Spark HealthCare, outside the submitted work; G. Ullrich: personal fees from Chiesi Pharma GmbH, personal fees from Novartis Pharma GmbH, personal fees from Gilead Sciences GmbH, personal fees from GSK GmbH & Co. KG, personal fees from Activaero GmbH, personal fees from Axcan Pharma GmbH, outside the submitted work. S. Bojcin, M. Bryon, A. Duff, N. Kashirskaya, I. Sermet-Gaudelus, K.W. Southern, G. Taccetti, and S. Wolfe have no conflicts of interest to report.

Keywords

  • Cystic fibrosis
  • Multidisciplinary management
  • Standards of care

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